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Review
. 2021 Dec 1;33(6):648-656.
doi: 10.1097/MOP.0000000000001062.

Coronavirus disease 2019 in patients with inborn errors of immunity: lessons learned

Giorgia Bucciol  1   2 , Stuart G Tangye  3   4 , Isabelle Meyts  1   2
Affiliations
Review

Coronavirus disease 2019 in patients with inborn errors of immunity: lessons learned

Giorgia Bucciol et al. Curr Opin Pediatr. .

Abstract

Purpose of review: The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic has caused extreme concern for patients with inborn errors of immunity (IEIs). In the first 6 months of the pandemic, the case fatality rate among patients with IEIs resembled that of the general population (9%). This review aims at summarizing what we have learned about the course and outcome of coronavirus disease 2019 (COVID-19) in patients with different IEIs and what this can potentially teach us about the immune mechanisms that could confer protection or predisposition to severe disease.

Recent findings: A total of 649 patients with IEI and COVID-19 have been reported in the last year and a half, spanning all groups of the International Union of Immunological Societies classification of IEIs. For most patients, the underlying IEI does not represent an independent risk factor for severe COVID-19. In fact, some IEI may even be protective against the severe disease due to impaired inflammation resulting in less immune-mediated collateral tissue damage.

Summary: We review the characteristics of SARS-CoV-2 infection in a large number of patients with IEI. Overall, we found that combined immunodeficiencies, immune dysregulation disorders, and innate immune defects impairing type I interferon responses are associated with severe disease course.

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Conflict of interest statement

I.M. is supported by the CSL Behring Chair in Primary Immunodeficiency in Children, paid to Institution. There are no conflicts of interest for the remaining authors.

Figures

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References

    1. Zhang Q, Bastard P, Liu Z, et al. . Inborn errors of type I IFN immunity in patients with life-threatening COVID-19. Science 2020; 370:eabd4570. - PMC - PubMed

    2. The article demonstrates the presence of inborn errors of type I interferon immunity in a subgroup of patients with critical coronavirus disease 2019 (COVID-19).

    1. Zhang Q, Bastard P, Bolze A, et al. . Life-threatening COVID-19: defective interferons unleash excessive inflammation. Med (New York, NY) 2020; 1:14–20. - PMC - PubMed

    2. The article summarizes the findings of the COVID Human Genetic Effort consortium on the critical role of type I interferon in severe acute respiratory syndrome coronavirus 2 infection and the genetic or acquired errors of interferon responses that underlie severe disease in a large number of patients.

    1. Lee PY, Platt CD, Weeks S, et al. . Immune dysregulation and multisystem inflammatory syndrome in children (MIS-C) in individuals with haploinsufficiency of SOCS1. J Allergy Clin Immunol 2020; 146:1194–1200.e1. - PMC - PubMed
    1. Chou J, Platt CD, Habiballah S, et al. . Mechanisms underlying genetic susceptibility to multisystem inflammatory syndrome in children (MIS-C). J Allergy Clin Immunol 2021; 148:732–738.e1. - PMC - PubMed
    1. Meyts I, Bucciol G, Quinti I, et al. . Coronavirus disease 2019 in patients with inborn errors of immunity: an international study. J Allergy Clin Immunol 2021; 147:520–531. - PMC - PubMed

    2. The article was the first and largest global survey of the presentation and outcome of COVID-19 in patients with inborn errors of immunity (IEIs).

Publication types